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Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial (ETFDH)

UniProt Number: Q16134
Alternate Names: Electron-transferring-flavoprotein dehydrogenase, ETFDH, ETFQO
Structure and Function: ETFDH or Electron-transferring-flavoprotein dehydrogenase in the inner mitochondrial membrane accepts electrons from electron-transfer flavoprotein which is located in the mitochondrial matrix and reduces ubiquinone in the mitochondrial membrane. The protein is synthesized as a 67-kDa precursor which is targeted to mitochondria and processed in a single step to a 64-kDa mature form located in the mitochondrial membrane. Deficiency in electron-transferring-flavoprotein dehydrogenase have been demonstrated in some patients with type II glutaricacidemia. ETFDH accepts electrons from ETF and reduces ubiquinone.
Disease Associations: Defects in ETFDH are the cause of glutaric aciduria type 2C (GA2C) [MIM:231680]. GA2C is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids.

Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount
ab126576 ETFDH antibody Mouse, Rat, Cow, Human WB 100 µg
ab131376 ETFDH antibody Mouse, Rat, Cow, Human ICC/IF 100 µg

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